According to a publication from ANCA Vasculitis News, a recent Spanish study of over 100 individuals diagnosed with ANCA-associated vasculitis found that major infections are a main cause of death among those with the disease.
About ANCA-Associated Vasculitis
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of rare diseases characterized by the inflammation and progressive destruction of small blood vessels running throughout the body.
The inflammation characteristic of this condition is caused by the activity of antineutrophil cytoplasmic antibodies — immune system proteins that attach to neutrophils (white blood cells) and make them attack friendly tissues like the walls of small or medium-sized blood vessels. Autoantibodies like ANCA, which can’t distinguish between foreign pathogens and friendly tissues, are responsible for a number of autoimmune disorders.
The symptoms and expression of ANCA vasculitis can change depending on where in the body flare-ups occur — skin attacks can result in rashes, while kidney attacks can cause blood to leak into the urine and disrupt important organ functions.
Reassessing the Threats of ANCA Vasculitis
In recent years, corticosteroids and immunosuppressive drugs have improved survival rates among ANCA-associated vasculitis patients. However, these treatments can have significant toxic side effects.
“…Nowadays treatment- related toxicity plays a central role in mortality,” a team of researchers from Spain noted of ANCA vasculitis patients. To determine just how central a role, the Spanish research team recruited 132 ANCA vasculitis patients for a study that would follow their general health for an average of twelve months.
Infection became increasingly common as autoimmune flare-ups occurred. In total, 79 of the 132 participants experienced major infections — with almost half of that group experiencing multiple. Those 79 individuals experienced 300 serious infections over the course of a year — almost four per person.
Infection is a major source of ANCA vasculitis-related deaths, cited as the cause of some two-thirds of AAV-related deaths that occur in the first year of diagnosis. In fact, the Spanish study found that mortality was so strongly correlated to infection that it was difficult to determine if the disease or the infection was causing these deaths. However, it was clear that while active disease was a main cause of mortality among AAV patients, researchers noted that “infectious events play a key role in the prognosis throughout the disease course.”
Although various subtypes of ANCA-associated vasculitis were represented in different proportions (51 had microscopic polyangiitis, 52 had granulomatosis with polyangiitis, and 29 had eosinophilic granulomatosis with polyangiitis), researchers found that there was no difference in infection rate between the groups.
By better identifying the factors contributing to patient mortality, physicians can improve the prognosis of AAV patients around the world.
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